[Case of von Recklinghausen disease complicated with a solitary lung lesion located mainly in the upper lung field].

A 72-year-old man presented with grade III dyspnea according to the Hugh-Jones scale in February, 2007, and he was referred to our department. Massive fibrosis of upper lung field dominance and bilateral pleural effusion were observed on chest X-ray films and CT. A respiratory function test revealed mixed ventilatory disturbance. Thereafter, left-sided pneumothorax developed in February 2008, followed by right-sided pneumothorax. He recovered once; however, respiratory failure progressed. He was re-hospitalized due to pneumonia and CO2 narcosis. Respiratory failure could not be prevented, and he died in September 2008. Macroscopic autopsy findings included fibrous adhesion of the pleura and fibrous consolidation of lung parenchyma which was most dominant in the bilateral apices. These were accompanied by bronchiectasis and brochiolectasis. Microscopically, the core pathology was organizing bronchiolitis and organizing pneumonia. Reported cases of diffuse lung lesions complicated with von Recklinghausen disease mostly comprise fibrosis and emphysematous changes. The unique pathological findings in this case seemed unrelated to any known disease entity.

Ability of high-resolution computed tomography to distinguish Mycoplasma pneumoniae pneumonia from other bacterial pneumonia: Significance of lateral bronchial lesions, less air bronchogram, and no peripheral predominance.

BACKGROUND: No study has investigated the capability of high-resolution computed tomography (HRCT) to detect a lateral bronchus abnormality, degree of air bronchogram, and distribution of affected lesions in the diagnosis of Mycoplasma pneumoniae pneumonia (MPP). METHODS: We prospectively enrolled patients with serologically-confirmed MPP or culture-confirmed other bacterial pneumonia (OBP). The distribution of affected areas, abnormalities in lateral bronchial lesions, the degree of air bronchogram, and previously reported findings on HRCT were evaluated for MPP and OBP. Predictive HRCT findings for MPP were determined by logistic regression analysis. We provisionally designed our HRCT criteria (negative, probable, or highly suspected) for diagnosing MPP and investigated the diagnostic yield of the HRCT criteria. RESULTS: Sixty-three MPP and 126 OBP patients were included in this study. Logistic regression analysis showed that the absence of peripheral predominance, bronchial wall thickening, lateral bronchial wall thickening, intralobular or lobular ground-glass opacities, intralobular ground-glass opacities connected to a lateral bronchus, and less air bronchogram in infiltrates were significant predictors of MPP. Our HRCT criteria showed that the sensitivity and specificity in negative, probable, and highly suspected MPP were 0.0 and 0.33, 1.0 and 0.69, and 0.5 and 0.98, respectively. CONCLUSIONS: HRCT had considerable ability to detect a lateral bronchial abnormality and to diagnose or rule out MPP based on the distribution of affected areas, abnormalities in lateral bronchial lesions, and the degree of air bronchogram in the infiltrates.

Adherence with long-term asthma management in patients who experienced hospitalized asthma exacerbation.

BACKGROUND: In Japan, the number of asthma deaths has been gradually decreasing. However, in the management of asthma, there are still some problems originating from patient-related factors and iatrogenic factors, both of which should be further analyzed. METHODS: We investigated clinical and background characteristics of 164 patients with asthma who were admitted to our hospital with acute exacerbations, by reviewing their clinical records. RESULTS: Fifty-two patients had received long-term management (LTM) based on the guidelines (the LTM group), while 112 had not (the non-LTM group). In patients whose asthma severity had been intermittent (step 1), the proportion of severe and near fatal exacerbations was significantly higher in the non-LTM group than in the LTM group. However, even in the LTM-group, 23% of mild persistent (step 2) and 38% of moderately and severely persistent (step 3 & 4) patients had severe or near fatal exacerbations. In these patients, the peak expiratory flow rate significantly improved after discharge, and poor adherence was also significantly higher in the non-LTM group than in the LTM group. A multivariate analysis revealed that the factors associated with poor adherence were: 1) no history of previous admission due to asthma exacerbation; 2) the patient was male; and 3) the patient was young (<60 years). CONCLUSIONS: In the LTM group, re-evaluation of the actual severity of asthma and prompt treatment corresponding to the severity of disease should still be encouraged. In the non-LTM group, establishing countermeasures against factors causing poor adherence would be the next step in ensuring strong adherence with LTM.

[A case of radiation pneumonitis with eosinophilia in bronchoalveolar lavage fluid].

A 78-year-old man was admitted to our hospital for irradiation therapy of non-resectable primary lung squamous cell carcinoma of the right middle lobe (T3N2M0). The Linac irradiation through opposing 2 gates (2Gy per day and 60Gy in total) was performed to the affected area including the metastatic right hilar and mediastinal lymphadenopathy. One week after completing the irradiation therapy, fever developed with infiltrates in the area from the right middle lobe to the right lower lobe, which did not necessarily coincide with the irradiated area. Antibiotic therapies were not effective. Both the serum LDH level and eosinophil count in the peripheral blood increased. Bronchoalveolar lavage was performed at the right B8, and differential cell counts of the lavage fluid were: macrophages, 17%; lymphocytes, 60%; neutrophils, 5%; and eosinophils, 18%. No significant organisms were obtained by culture of the lavage fluid. The %VC and DLCO/VA became lower than before the irradiation therapy. Thus, the patient was given a diagnosis of radiation pneumonitis. Treatment with 40mg/day oral prednisolone was commenced with a stepwise dose-reduction (5mg every two weeks) until reaching the maintenance dose of 15mg/day. The serum LDH level and blood eosinophil count recovered promptly to the normal range. The pulmonary infiltrates and the lung functions substantially improved. There have been few reports of radiation pneumonitis in which eosinophil counts increased in peripheral blood and bronchoalveolar lavage fluid after irradiation therapy. In the present case report, the possible mechanisms for the irradiation-induced eosinophilia were also reviewed.

Phenotypic analysis of vertigo 2 Jackson mice with a Kcnq1 potassium channel mutation.

The KCNQ1 gene encodes a voltage-dependent potassium ion channel, and mutations in this gene are the most common cause of congenital long QT syndrome (LQTS). In the present study, we investigated the various phenotypic characteristics of vertigo 2 Jackson (C3H/HeJCrl-Kcnq1(vtg-2J)/J) mice with a Kcnq1 mutation. Both heterozygotes (vtg-2J/+) and homozygotes (vtg-2J/vtg-2J) showed prolonged QT intervals in electrocardiograms (ECGs) compared to C3H/HeJ control (+/+) mice. Furthermore, vtg-2J/vtg-2J mice showed gastric achlorhydria associated with elevation of their serum gastrin levels. The serum corticosterone levels were also significantly increased in vtg-2J/vtg-2J mice. In addition, vtg-2J/vtg-2J mice exhibited significantly higher blood pressure. These findings indicate that the Kcnq1 mutation in vtg-2J mice alters various physiological functions in the cardiac, gastric and adrenocortical systems, and suggest that vtg-2J mice may represent a useful model for studying Kcnq1 functions.

Visceral larva migrans due to Ascaris suum which presented with eosinophilic pneumonia and multiple intra-hepatic lesions with severe eosinophil infiltration--outbreak in a Japanese area other than Kyushu.

A 32-year-old man presented with the chief complaint of severe cough. Examination of peripheral blood showed a marked increase in eosinophils. Chest CT demonstrated multiple ground glass opacities in both lungs. Bronchoalveolar lavage showed abundant eosinophils. Abdominal CT demonstrated multiple low attenuation areas in the liver. Liver biopsy with ultrasonography revealed severe eosinophil infiltrations around the portal veins. Serologically, a multi-dot enzyme linked immunosorbent assay (DOT-ELISA) and ELISA inhibition test using microtiter plates were positive for Ascaris suum. Thus, visceral larva migrans due to Ascaris suum was diagnosed. Outbreaks of this disease in Japan have previously been confined to the Kyushu area. The present case which occurred outside that area, illustrates the importance of constant attention to the epidemiology of this disease.

Predictive values of semi-quantitative procalcitonin test and common biomarkers for the clinical outcomes of community-acquired pneumonia.

BACKGROUND: The semi-quantitative serum procalcitonin test (Brahms PCT-Q) is available conveniently in clinical practice. However, there are few data on the relationship between results for this semi-quantitative procalcitonin test and clinical outcomes of community-acquired pneumonia (CAP). We investigated the usefulness of this procalcitonin test for predicting the clinical outcomes of CAP in comparison with severity scoring systems and the blood urea nitrogen/serum albumin (B/A) ratio, which has been reported to be a simple but reliable prognostic indicator in our prior CAP study. METHODS: This retrospective study included data from subjects who were hospitalized for CAP from August 2010 through October 2012 and who were administered the semi-quantitative serum procalcitonin test on admission. The demographic characteristics; laboratory biomarkers; microbiological test results; Pneumonia Severity Index scores; confusion, urea nitrogen, breathing frequency, blood pressure, ≥ 65 years of age (CURB-65) scale scores; and age, dehydration, respiratory failure, orientation disturbance, pressure (A-DROP) scale scores on hospital admission were retrieved from their medical charts. The outcomes were mortality within 28 days of hospital admission and the need for intensive care. RESULTS: Of the 213 subjects with CAP who were enrolled in the study, 20 died within 28 days of hospital admission, and 32 required intensive care. Mortality did not differ significantly among subjects with different semi-quantitative serum procalcitonin levels; however, subjects with serum procalcitonin levels ≥ 10.0 ng/mL were more likely to require intensive care than those with lower levels (P < .001). The elevation of semi-quantitative serum procalcitonin levels was more frequently observed in subjects with proven etiology, especially pneumococcal pneumonia. Using the receiver operating characteristic curves for mortality, the area under the curve was 0.86 for Pneumonia Severity Index class, 0.81 for B/A ratio, 0.81 for A-DROP, 0.80 for CURB-65, and 0.57 for semi-quantitative procalcitonin test. CONCLUSIONS: The semi-quantitative serum procalcitonin level on hospital admission was less predictive of mortality from CAP compared with the B/A ratio. However, the subjects with serum procalcitonin levels ≥ 10.0 ng/mL were more likely to require intensive care than those with lower levels.

Prognostic value of severity indicators of nursing-home-acquired pneumonia versus community-acquired pneumonia in elderly patients.

BACKGROUND: The credibility of prognostic indicators in nursing-home-acquired pneumonia (NHAP) is not clear. We previously reported a simple prognostic indicator in community-acquired pneumonia (CAP): blood urea nitrogen to serum albumin (B/A) ratio. This retrospective study investigated the prognostic value of severity indicators in NHAP versus CAP in elderly patients. METHODS: Patients aged ≥65 years and hospitalized because of NHAP or CAP within the previous 3 years were enrolled. Demographics, coexisting illnesses, laboratory and microbiological findings, and severity scores (confusion, urea, respiratory rate, blood pressure, and age ≥65 [CURB-65] scale; age, dehydration, respiratory failure, orientation disturbance, and pressure [A-DROP] scale; and pneumonia severity index [PSI]) were retrieved from medical records. The primary outcome was mortality within 28 days of admission. RESULTS: In total, 138 NHAP and 307 CAP patients were enrolled. Mortality was higher in NHAP (18.1%) than in CAP (4.6%) (P<0.001). Patients with NHAP were older and had lower functional status and a higher rate of do-not-resuscitate orders, heart failure, and cerebrovascular diseases. The NHAP patients more frequently had typical bacterial pathogens. Using the receiver-operating characteristics curve for predicting mortality, the area under the curve in NHAP was 0.70 for the A-DROP scale, 0.69 for the CURB-65 scale, 0.67 for the PSI class, and 0.65 for the B/A ratio. The area under the curve in CAP was 0.73 for the A-DROP scale, 0.76 for the CURB-65 scale, 0.81 for the PSI class, and 0.83 for the B/A ratio. CONCLUSION: Patient mortality was greater in NHAP than in CAP. Patient characteristics, coexisting illnesses, and detected pathogens differed greatly between NHAP and CAP. The existing severity indicators had less prognostic value for NHAP than for CAP.

Impact and indication of early systemic corticosteroids for very severe community-acquired pneumonia.

BACKGROUND: The efficacy of systemic corticosteroids in community-acquired pneumonia (CAP) has not yet been confirmed. We prospectively investigated the clinical features of patients treated with early adjunctive systemic corticosteroids and its clinical impact in very severe CAP. METHODS: One hundred and one consecutive CAP patients having a pneumonia severity index of >130 points were enrolled from August 2010 through February 2013. Early adjunctive systemic corticosteroids were defined as administration of systemic corticosteroids equivalent to prednisone of ≥20 mg/day added to initial antibiotics. The multivariate analysis was performed to evaluate the independent factors associated with mortality. RESULTS: Thirty-two patients (31.7%) died within 28 days of admission. Early adjunctive systemic corticosteroids were administered in 30 patients (29.7%), who more frequently had alteration of mental status, serious respiratory failure, or underlying lung diseases and received fluoroquinolones as initial antibiotics. In most patients treated with early adjunctive systemic corticosteroids, the dosage was less than 60 mg/day of an equivalent to prednisone by bolus intravenous infusion for a period shorter than 8 days. The occurrence of adverse events did not differ between the groups. Factors independently associated with mortality were blood urea nitrogen (hazard ratio [HR] 1.02, 95% confidence interval [CI] 1.00-1.04), serum albumin (HR 0.44, 95% CI 0.22-0.86), a requirement for intensive care (HR 4.93, 95% CI 1.75-13.87), and the therapy with early adjunctive systemic corticosteroids (HR 0.29, 95% CI 0.11-0.81). CONCLUSION: Early adjunctive systemic corticosteroids may have an effect to reduce the mortality in very severe CAP, although a larger-scale study is necessary.

Blood urea nitrogen to serum albumin ratio independently predicts mortality and severity of community-acquired pneumonia.

BACKGROUND: Early studies of community-acquired pneumonia showed that nonsurvivors had higher blood urea nitrogen levels and lower serum albumin levels than survivors. Therefore, elevation of the blood urea nitrogen to serum albumin (B/A) ratio may identify patients with community-acquired pneumonia who are becoming critically ill. This study investigated the correlation between commonly used laboratory markers, in particular the B/A ratio, and clinical outcomes of community-acquired pneumonia. METHODS: This observational study was performed in consecutive patients with community-acquired pneumonia admitted to our hospital over a period of one year. Blood counts, commonly used laboratory markers, microbiological tests, and calculation of Pneumonia Severity Index (PSI) and CURB-65 were done on admission. The endpoints were mortality within 28 days of admission and requirement for intensive care. RESULTS: One hundred and seventy-five patients with community-acquired pneumonia were enrolled. Nineteen patients died within 28 days of admission and 29 patients required intensive care. Using multivariate analysis, independent factors associated with mortality were the requirement for intensive care (odds ratio [OR] 14.96, 95% confidence interval [CI] 3.73-60.03, P < 0.001), PSI class (OR 3.55, 95% CI 1.08-11.66, P = 0.037), and B/A ratio (OR 1.10, 95% CI 1.01-1.20, P = 0.037). Similarly, independent factors associated with need for intensive care were PSI class (OR 5.35, 95% CI 1.90-15.06, P = 0.002), CURB-65 (OR 2.37, 95% CI 1.26-4.45, P = 0.007), and B/A ratio (OR 1.27, 95% CI 1.09-1.47, P = 0.002). CONCLUSION: The B/A ratio is a simple but independent predictor of mortality and severity of community-acquired pneumonia.

Age-dependent deterioration of peak inspiratory flow with two kinds of dry powder corticosteroid inhalers (Diskus and Turbuhaler) and relationships with asthma control.

BACKGROUND: Inhaled corticosteroid (ICS) therapy has improved the quality of life (QOL) for many asthmatics and reduced mortality rates associated with asthma. However, some patients do not obtain therapeutic benefit despite satisfactory adherence. OBJECTIVES: To determine whether asthmatic patients were using ICS devices appropriately, and to clarify relationships between these results and QOL. SUBJECTS AND METHODS: We studied 100 adult asthmatics, divided into two groups: 50 patients consecutively registered as using Diskus (fluticasone; D-group) and 50 consecutively registered as using Turbuhaler (budesonide; T-group). We measured peak inspiratory flows (PIFs) using the In-Check Dial device. Subjects also completed the Asthma Control Test for evaluation of QOL. RESULTS: In the D-group, no patients showed PIF below the optimal range (30-90 L/min), whereas 52% of patients had PIF≥91 L/min. In the T-group, 6% of patients showed PIF over the optimal range (60-90 L/min), and 44% had PIF≤59 L/min. When patients in the T-group were required to deliberately make a maximal inhalation, 14% still had PIF≤59 L/min. The proportion of patients with poor control was significantly greater in the T-group than in the D-group. According to univariate logistic regression analyses, low PIF tended to be associated with poor asthma control in the T-group. No significant correlation was found between PIF and age in the D-group, but PIF decreased significantly with age in the T-group. CONCLUSIONS: Appropriate measures for patients in whom PIF has been judged as lower than optimal include adequate education for inhalation and/or changing to a different inhalation device. These measures should be kept in mind for elderly asthma patients in particular, where appropriate selection of a corticosteroid inhalation device in the early stages of therapy would also be important.

Long-term observations of the clinical course after step down of corticosteroid inhalation therapy in adult chronic asthmatics: correlation with serum levels of eosinophil cationic protein.

BACKGROUND: Symptoms often deteriorate in well-controlled asthmatics after a step down in inhaled beclomethasone dipropionate (iBDP) therapy if the serum concentration of eosinophil cationic protein (sECP) is high. This deterioration is significantly abrogated by pranlukast, a leukotriene receptor antagonist, or by seratrodast, a thromboxane A2 receptor antagonist. However, these results were based on short-term (less than 6 months) observations. METHODS: We studied 35 well-controlled adult asthmatics. We assigned the patients into different groups according to their sECP levels before the step down: (i) group A, sECP < 25 microg/L; (ii) group B, sECP > or = 25 microg/L; and (iii) group C, sECP > or = 25 microg/L but patients treated with pranlukast or seratrodast. We began the study with a step down in iBDP therapy (initial step down), then followed the clinical course of the asthma for 2 years. During the study period, we decreased, increased or maintained the iBDP dose on the basis of the stepwise approach described in the National Institutes of Health guidelines. We monitored the time and frequency of exacerbation and evaluated the iBDP dose required to control the asthma symptoms. RESULTS: The rates of exacerbation after the step down were high in groups A and B. In group A, the conditions were again qualified for the step down in all patients, but this was not the case for most group B patients. From 15 to 21 months after the initial step down, the average dose of iBDP required to control symptoms was significantly higher in group B than in group A patients (P = 0.0127-0.0373). The exacerbation rate in group C after 12 months tended to be lower than in the other two patient groups (P = 0.0743). In group C, the average dose of iBDP from 9 to 24 months after the initial step down was significantly lower than before the step down (P < 0.0001) and was not significantly different from the mean dose of iBDP in groups A or B. CONCLUSIONS: High sECP in well-controlled asthma may indicate the necessity for a higher iBDP dose over a long period than when the sECP concentration is not high. Even if sECP is high, pranlukast or seratrodast help to prevent exacerbation of asthma and enable successful step down in iBDP therapy for at least 2 years thereafter.